Hypospadias repair

Definition

Hypospadias repair refers to a group of surgical approaches used to
correct or reconstruct parts of the external genitalia and urinary tract
related to a displaced meatus, or opening of the urethra. The urethra is
the passageway that carries urine from the bladder to the outside of the
body. Hypospadias is the medical term for a birth defect in which the
urethra opens on the underside of the penis (in boys) or into the vagina
(in girls). The word hypospadias comes from two Greek words that mean
underneath and rip or tear, because severe forms of hypospadias in boys
look like large tears in the skin of the penis.

Hypospadias is one of the most common congenital abnormalities in males.
It was described in the first and second centuries
A
.
D
. by Celsus, a Roman historian of medicine, and Galen, a Greek physician.
The first attempt to correct hypospadias by surgery was made in 1874 by
Duplay, a French surgeon; as of 2003, more than 200 different procedures
for the condition have been reported in the medical literature.

Hypospadias repair is, however, controversial because it is genital
surgery. Some people regard it as unnecessary interference with a
child's body and a traumatic experience with psychological
consequences extending into adult life. Others maintain that boys with
untreated hypospadias are far more likely than those who have had surgery
to develop fears about intimate relationships and sexuality. There is
little information about the emotional aftereffects of hypospadias repair
on girls.

Purpose

Although there are several different surgical procedures used at present
to correct hypospadias depending on its severity, all have the following
purposes:

To permit emptying of the bladder standing up. The abnormal location of
the urethral meatus on the underside of the penis forces many boys to
void urine sitting down, which leads to anxiety about using public
restrooms or otherwise being seen undressed by other males.

To correct a condition associated with hypospadias known as chordee.
Chordee, which comes from the French
cordée
, which means tied or corded, is a condition in which the penis bends
downward during an erection. This curving or bending makes it difficult
to have normal sexual intercourse as an adult.

To prevent urinary tract infections (UTIs). It is common in hypospadias
for the urethral meatus to be stenotic, or abnormally narrowed. A
stenotic urethra increases the risk of frequent UTIs.

To lower the risk of developing testicular cancer. Hypospadias has been
identified as a risk factor for developing testicular cancer after
adolescence.

To confirm the boy's sexual identity by improving the outward
appearance of the penis. The external genitals of babies with severe
hypospadias may look ambiguous at birth, causing stress for the parents
about their child's gender identity.

Demographics

Hypospadias is much more common in males than in females. In Canada and
the United States, the incidence of hypospadias in boys is estimated to be
1:250 or 1:300 live births. In girls, the condition is very rare,
estimated at 1:500,000 live births. One troubling phenomenon is the
reported doubling of cases of hypospadias in both Europe and North America
since the 1970s without any obvious explanation. According to a recent
press release from the U.S. Centers for Disease Control and Prevention
(CDC), data from two surveillance systems monitoring birth defects in the
United States show that the rate of hypospadias rose from 36 per 10,000
male births in 1968 to 80 per 10,000 male births in 1993. In addition to
the increase in the number of cases reported, the proportion of severe
cases has also risen, which means that the numerical increase cannot be
explained as the result of better reporting.

The severity of hypospadias is defined according to the distance of the
urethral opening from its normal location at the tip of the penis. In mild
hypospadias, which is sometimes called coronal/glandular hypospadias, the
urethral opening is located on the shaft of the penis just below the
glans. In mild to moderate hypospadias, the opening is located further
down the shaft of the penis toward the scrotum. In severe hypospadias,
which is also called penoscrotal hypospadias, the urethral opening is
located on the scrotum. About 80–85% of hypospadias are classified
as mild; 10–15% as mild to moderate; and 3–6% as severe.

In hypospadias, the urethral opening is at the base of the penis,
instead of the tip (A). Tissue grafts are used to create an
extension for the urethra (C) and alleviate the tight skin, or
chordee, on the underside of the penis.
(

Illustration by GGS Inc.

)

Although the causes of hypospadias are not yet fully understood, the
condition is thought to be the end result of a combination of factors. The
following have been associated with an increased risk of hypospadias:

Genetic inheritance. Hypospadias is known to run in families; a boy with
hypospadias has a 28% chance of having a male relative with the
condition.

Genetic disorders. Hypospadias is found in boys with a deletion on human
chromosome 4p, also known as Wolf-Hirschhorn syndrome; and in persons
with a variety of intersex conditions related to chromosomal
abnormalities. Several different genetic mutations responsible for a
deficiency in 5-alpha reductase, an enzyme needed to convert
testosterone to a stronger androgen needed for urethral development,
have been found in boys with hypospadias.

Low birth weight. Several studies in the United Kingdom as well as in
the United States have shown that
male infants with hypospadias weigh less and are smaller at birth than
controls. It is thought that these low measurements are markers of fetal
androgen dysfunction.

Drugs taken by the mother during pregnancy. Diethylstilbestrol (DES), a
synthetic hormone that was prescribed for many women between 1938 and
1971 to prevent miscarriage, has been associated with an increased risk
of stenosis of the urethral meatus as well as hypospadias in the sons of
women who took the medication. Boys born to mothers addicted to cocaine
also have an abnormally high rate of hypospadias.

Environmental contamination. One proposal for explaining the rising rate
of hypospadias and other birth defects in males is the so-called
endocrine disruptor hypothesis. Many pesticides, fungicides, and other
environmental pollutants contain estrogenic or anti-androgenic
substances that interfere with the normal androgen pathways in embryonic
tissue development—in birds and other animals as well as in
humans.

Assisted reproduction. A study done in Baltimore of children who were
conceived through
in vitro fertilization
(IVF) between 1988 and 1992 found that the incidence of hypospadias
among the males was five times that of male infants in a control group.

With regard to ethnic and racial differences in the American population,
the CDC reports that Caucasians have the highest rates of hypospadias,
Hispanics have the lowest, and African Americans have intermediate rates.
Other studies have found that hypospadias is more common in males of
Jewish or Italian descent than in other ethnic groups.

Description

Correction of hypospadias in boys

The specific surgical procedure used depends on the severity of the
hypospadias. The objectives of surgery always include widening the
urethral meatus; correcting chordee, if present; reconstructing the
missing part of the urethra; and making the external genitalia look as
normal as possible. Most repair procedures take between one-anda-half and
three hours, and are performed under general anesthesia. Mild hypospadias
can be corrected in a one-step procedure known as a meatal advancement and
glanduloplasty, or MAGPI. In a MAGPI procedure, the opening of the urethra
is moved forward and the head of the penis is reshaped. More severe
hypospadias can also be corrected in one operation, which involves
degloving the penis (separating the skin from the shaft) in order to cut
the bands of tissue that cause chordee, and constructing a new urethra
that will reach to the tip of the penis. The specific technique of
reconstruction is usually decided in the
operating room
, when the surgeon can determine how much tissue will be needed to make
the new urethra. In some cases, tissue must be taken from the inner arm or
the lining of the mouth. In a few cases, the repair may require two or
three stages spaced several months apart.

There is some remaining disagreement among professionals regarding the
best age for hypospadias repair in boys. Most surgeons think the surgery
should be done between 12 and 18 months of age, on the ground that gender
identity is not fully established prior to toilet training and the child
is less likely to remember the operation. Some doctors, however, prefer to
wait until the child is about three years old, particularly if the repair
involves extensive reconstruction of the urethra.

Recent advances in hypospadias repair include the use of tissue glues and
other new surgical adhesives that speed healing and reduce the risk of
fistula formation. In addition, various synthetic materials are being
tested for their suitability in constructing artificial urethras, which
would reduce the risk of complications related to
skin grafting
.

Correction of hypospadias in girls

The most common surgical technique for correcting hypospadias in girls is
construction of a new urethra that opens to the outside of the body rather
than emptying into the vagina. Tissue is taken from the front wall of the
vagina for this purpose.

Diagnosis/Preparation

Diagnosis

The diagnosis of hypospadias in boys is often made at the time of delivery
during the newborn examination. The condition may also be diagnosed before
birth by ultrasound; according to a group of Israeli researchers,
ultrasound images of severe hypospadias resemble the outline of a tulip
flower. Ultrasound is also used prior to surgical repair to check for
other abnormalities, as about 18% of boys with hypospadias also have
cryptorchidism (undescended testicles), inguinal hernia, or defects of the
upper urinary tract.

Hypospadias in girls may not be discovered for several months after birth
because of the difficulty of examining the vagina in newborn females.

Preparation

Male infants with hypospadias should not be circumcised as the foreskin
may be needed for tissue grafting during repair of the hypospadias.

Some surgeons prescribe small doses of male hormones to be given to the
child in advance to increase the size of the penis and improve blood
supply to the area. The child may also be given a mild sedative
immediately before surgery to minimize memories of the procedure.

Aftercare

Short-term aftercare

Many anesthesiologists provide a penile nerve block to minimize the
child's postoperative discomfort. Dressings are left in place for
about four days. The surgeon places a stent, which is a short plastic tube
held in place with temporary stitches, or a catheter to keep the urethra
open. The patient is usually given a course of
antibiotics
to reduce the risk of infection until the dressings and the stent or
catheter are removed, usually 10–14 days after surgery.

The child should be encouraged to drink plenty of fluids after returning
home in order to maintain an adequate urinary output. Periodic follow-up
tests of adequate urinary flow are typically scheduled for three weeks,
three months, and 12 months after surgery.

Long-term aftercare

Boys who have had any type of hypospadias repair should be followed
through adolescence to exclude the possibility of chronic inflammation or
scarring of the urethra. In some cases, psychological counseling may also
be necessary.

Risks

In addition to the risks of bleeding and infection that are common to all
operations under general anesthesia, there are some risks specific to
hypospadias repair:

Wound dehiscence. Dehiscence means that the incision splits apart or
reopens. It is treated by a follow-up operation.

Bladder spasms. These are a reaction to the presence of a urinary
catheter, and are treated by giving medications to relax the bladder
muscles.

Fistula formation. A fistula is an abnormal opening that forms between
the reconstructed urethra and the skin. Most fistulae that form after
hypospadias surgery close by themselves within a few months. The
remainder can be closed surgically.

Urethral stenosis. Narrowing of the urethral opening after surgery is
treated by dilating the meatus with urethral probes.

Normal results

Hypospadias repair in both boys and girls has a high rate of long-term
success. In almost all cases, the affected children are able to have
normal sexual intercourse as adults, and almost all are able to have
children.

Morbidity and mortality rates

Surgical repair of hypospadias has a fairly high short-term complication
rate:

leakage of urine from the area around the urethral meatus: 3–9%

formation of a fistula: 0.6–23% for one-stage procedures;
2–37% for two-stage procedures

urethral stenosis: 8.5%

persistent chordee: less than 1%

Alternatives

There are no medical treatments for hypospadias as of 2003. The only
alternative to surgery in childhood is postponement until the child is old
enough to decide for himself (or herself) about genital surgery.

WHO PERFORMS THE PROCEDURE AND WHERE IS IT PERFORMED?

Surgery to correct hypospadias is done by a pediatric urologist, a surgeon
with advanced training in urology as well as in treating disorders
affecting children. According to the Society for Pediatric Urology (SPU),
pediatric urologists educated in the United States have completed two
years in a
general surgery
residency after medical school, followed by four years in a
urologic surgery
residency and an additional two years in a pediatric urology fellowship
program.

Surgical procedures to correct mild or mild to moderate hypospadias with
little chordee may be done on an outpatient basis. Correction of moderate
or severe hypospadias with some chordee, however, involves hospitalizing
the child for 1–2 days. Parents can usually arrange to stay
overnight with their child.

QUESTIONS TO ASK THE DOCTOR

How often do you perform hypospadias repair, and what is your success
rate?

How severe is my child's hypospadias, and what procedure do you
recommend to correct it?

What do you consider the best age for corrective genital surgery and
why?

in case of hypospadia not operated at childhood,he is married one and half year ago;his wife had no pregnancy during this period;his wife investigations were normal ;but he had less than12.5%rapid progressive sperm ;what is the cause of infirtility....hypospadia or low active rapid sperm level??!!

thank you very much for this information.my son underwent an hypospadiasis operation in 2008 in india . now a sack is formed under his penis. urine got collected in that sack and stone has formed. the stone was removed after surgery.the cathiter was blcked after 3 days they changed the dressing and removed the cathiter.now there is a tear on his stitch. will you be able to help me out in this.

I have been in the end of 2008 & suffering from penoscrotal hypospadias. All is well but formed fistulae and semen is not coming out of the constructed meatus. Urine is
Coming always but sometimes after few seconds late. Guide me Plz?

gud day..im have a hypospadia..severe one..im already 20 years old now..im wondering if theres a way to fix my defect...my parents here in the Philippines doesnt know about my situation..perhaps thats the reason why they just let my unusual penis be as it is..

my son is already 13 and is about to have his circumcision when we found out about this defect, his doctor did not proceed with it instead we are reffered to a urologist, now we have to prepare for a major operation,it will be very expensive since it was found already in the late stage already.thank you for the information.atleast now it has opened my mind how to cope with situation.

Kindly my son has Hypospidas case and he did the operation from one and half month and he has a fistula could you please advice after how many month we can say that its a mature fistula that needs an operation to be fixed by.

Kindly my son has Hypospidas case and he did the operation from one and half month and he has a fistula could you please advice after how many month we can say that its a mature fistula that needs an operation to be fixed by.

I am 42 and have been thinking of having the hypospadias repaired. Can you tell me more about this? When I urinerate it splatters everywhere so I usually sit to do this. I would love to be able to stand and urinerate. It is about a half an inch opening. I have never talked about this to my parents and they me either. There has been some sugery at birth, half of my foreskin has been removed and stitch marks all the way down the shaft of my penis. Can you give me any answers and advise?

YA I HAD A HYPO SURGERY WHEN I WAS BORN IT HAS AFFECTED ME IN MANY WAYS. IT WAS A VERY SERIOUS PROBLEM. I WOULD LIKE TO STAND AND SAY I HAVE ACHIEVED MANY RESULTS IN MY LIFE. I WAS IN THE 82ND AIRBORNE INFANTRY PARACHUTE AND I DID ALMOST 20 YRS OF MACHINING WORK. NOW IM A COMMERCIAL DIVER. I WISH I CAN HELP. I HAVE SO MANY STORY TO TELL. IF I COULD ELABORATE ITS HARD TO BELIEVE MY FAMILY AND CIRCUMSTANCES

iam 29 year getting marriage...so please give me the solution for my problem, in my childhood i got hypospadisis surgery in MUMBAI...now i have the same problem,so is it possible to operation in this age..

i had hypospadiis surgery 10 years ago,still having issues with restrictions,,recently saw a urologist and took x-rays,found restrictions in my urinary tract ,is saying i need reconstuctive surgery to take care of concern, said he would need to take tissue from my mouth and possible bladder to complete, does this sound common,any advice would be appreicated

I had 5 repair surgeries from age 5 to 13. Everything works but it has caused severe psychological problems throughout my life. I am now 56 years old. I have an ugly penis which has kept me from searching out sexual partners for the past 14 years although I have had several partners throughout the years and no one has ever kicked me out of bed because of it. I don't like the way it looks and I would like to consider surgery for cosmetic reasons. I am also gay as if that has anything to do with it.
Where do I begin searching for the right surgeon? Should I contact a urologist and go from there?
Any replies will be appreciated.
Thank you, Don

nw i am going 2 30yr old.maried 2 yrs before.last week i found one drs talk in tv about hypospadias.then i found my hypospadias.its just below the glands.aftr erection its curved down.i have difficulty in sexual intercourse..can get corrected in this age group.its difficult or not.aftr correction i can maintain the normal sex.pls give me the advise.

I have a 15 month old grandson with severe hypospadius. His urethra emerges at the base of his penis and his penis is held in the downward position by a hood of skin. They have said that it will require two surgeries to correct it. His testicles are positioned on either side right next to his penis and he lacks a scrotum. His parents have been counseled that he should have that repaired as well but they are thinking that it is just cosmetic. Will he be sterile if that is not repaired due to the fact that a scrotum is reactive to heat and cold to keep the sperm at the right temperature ? I have heard that some men have trouble concieving if they wear tight briefs because the sperm die as the testicles are too hot. Also wouldn't his testicles be less protected from injury where they are now ? If they do have to repair that what skin do they graft that would be reactive to temperature ?

As I was reading this I realized that the reason the corrective surgery hasn't been performed on children since 2003, is because its considered cosmetic and or the laws regarding children needing corrective surgery due to un distinguished gender of the new born. My husband has this and underwent surgery as a baby. Has the world really become that complex that doctors can't tell if a child doesn't have a penis or is born with a birth abnormality? There is a woman worried about her grandson who was born without a scrotem, I'm not a doctor but I would have to say that that isn't cosmetic! We are talking about quality of life here! I understand the issue behind children born with no gender and the issues behind deciding a gender for a child through surgery. But that's not what this is! This is a child born with a known gender, with a birth defect that can be as severe as the tip of the penise split in two. Though the child isn't in pain and can use the bathroom, I don't think sex will be an option for that particular child as an adult. Here is my issue, I once read that this wasn't genetic but have since learned that it is. So if our son is born with hypospadios my insurance will not cover corrective surgery. Kinda makes a women have second thoughts about children, there for effecting the mans quality of life extreamly! Especially since my husband look forward to being a father.

i am 38yr old..last week i found one drs talk in tv about hypospadias.then i found my hypospadias.its just below the glands.aftr erection its curved down..can get corrected in this age group.its difficult or not.aftr correction i can maintain the normal sex.pls give me the advise.

I am a woman with this condition. Where can I go to get help for me and my family and coping, surgery. Where can I go! I am from NY and really need information about women with this condition. I understand it is quite rare, and would like to get help as I have symptoms to correct.

Hi, I am 33 years old Filipino and I am currently here in UAE, where in the Philippines can I found cheaper surgery for this, I am not wealthy person but I can save for the operation maybe 2-3 years from now. Please help me or is there any foundations that can help me with this? Thanks, God bless.

My son was diagnosed with an encased penis at birth. The doctor still felt like he was able to perform a circumcision and was able to release much of the skin around the glans. However he was still left with chordee. As he grew older we sought out opinions around age 8. The drs ranged from do nothing to surgery, the thought being it may be painful in intercourse later in life, which is why one would opt for surgery.

We opted for surgery, thinking it was a relatively minor procedure. We were misinformed on several levels and it was a huge mistake.

#1 You must seek out a top pediactric urologist or two to get their assesement for your son. If he is good our experience is you might have to wait weeks for an appt.

#2 If you end up having surgery, get informed about catethers, BEFORE the surgery.
i) Insist on the pediactric urologist fastening the catether to you son's body after surgery. No, a nurse or other medical staff member is NOT sufficient to do this.
ii) Triple check the catether before you leave the hospital - that is is draining and it is properly fastened to prevent kinking or pulling on the body.
iii) Continously monitor that the catheder is draining. No drainage for a couple hours could very well indicate it is kinked / plugged which is a very serious condition. You MUST monitor this carefully.
iv) If the catheder becomes plugged, go to the emergency room ASAP. If you are unable to do this, carefullly remove all tape on the catheder so you can visibly see all lengths of the catheder, to verify there are NO kinks in the tube outside of the body.
v) While at any appointment, (ex dressing removal) or in the hospital, you must ASK questions about what any medical staff / nurse is ABOUT to do to your son. You must then give the OK. It is our experience many are unfamiliar with catheders and any dressing/tape applied to them. They can be EASILY accidentally cut by nurses/staff that are not paying attention.

#3 If the chordee repair fails, scar tissue can build in the urethra and it can basically become plugged, forcing surgery, to removing the problem urethra area which then creates a hypospadius condition.

#4 If you have a self created hypospadius condition due to situations like #3, it could take multiple surgeries to reconstruct the urethra.

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